National networking in rare diseases and reduction of cardiac burden in thalassemia major.

AIMS: A tailored chelation therapy guided by magnetic resonance imaging (MRI) is a strategy to improve the prognosis in iron-loaded patients, in many cases still hampered by limited MRI availability. In order to address this issue, the Myocardial Iron Overload in Thalassemia (MIOT) network was established in Italy and we aimed to describe the impact of 10-year activity of this network on cardiac burden in thalassemia major (TM). METHODS AND RESULTS: Within the MIOT network, 1746 TM patients (911 females; mean age 31.2 ± 9.1 years) were consecutively enrolled and prospectively followed by 70 thalassemia and 10 MRI centres. Patients were scanned using a multiparametric approach for assessing myocardial iron overload (MIO), biventricular function, and myocardial fibrosis. At the last MRI scan, a significant increase in global heart T2* values and a significantly higher frequency of patients with no MIO (all segmental T2* ≥20 ms) were detected, with a concordant improvement in biventricular function, particularly in patients with baseline global heart T2* <20 ms. Forty-seven percentage of patients changed the chelation regimen based on MRI. The frequency of heart failure (HF) significantly decreased after baseline MRI from 3.5 to 0.8% (P < 0.0001). Forty-six patients died during the study, and HF accounted for 34.8% of deaths. CONCLUSION: Over 10 years, continuous monitoring of cardiac iron and a tailored chelation therapy allowed MIO reduction, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related HF. A national networking for rare diseases therefore proved effective in improving the care and reducing cardiac outcomes of TM patients.

LI-RADS ancillary features favoring benignity: is there a role in LR-5 observations?

OBJECTIVES: The Liver Imaging Reporting and Data System algorithm allows category downgrade in the presence of ancillary features (AFs) favoring benignity, even in observations categorized as LR-5. This study aims to assess the role of AFs favoring benignity in LR-5 observations and their impact on category downgrade. METHODS: This study included high-risk patients with at least one LR-5 observation imaged with gadoxetate disodium MRI. Three readers with different experience levels independently evaluated the presence of AFs favoring malignancy (not hepatocellular carcinoma (HCC) in particular and HCC in particular) and AFs favoring benignity. Category downgrade was considered possible in the presence of ≥ 1 AF favoring benignity and no AF favoring malignancy. Correlation between observations size and number of AFs was assessed using Spearman's rank correlation coefficient. Cohen's kappa (k) test was used to assess inter-reader agreement. RESULTS: The final study cohort included 162 LR-5 (mean size: 23 ± 16 mm) in 119 patients. AFs favoring benignity were reported in 9 (5.6%), 20 (12.3%), and 10 (6.2%) LR-5 observations by reader 1, reader 2, and reader 3, respectively. Hepatobiliary phase isointensity was observed in 6 (3.7%), 2 (1.2%), and 7 (4.3%) observations, respectively. Category downgrade was considered possible in only one (0.6%) observation by reader 1 and reader 3. There was a significant correlation between observation size and number of AFs favoring malignancy (p < 0.001), not HCC in particular (p ≤ 0.010), and favoring HCC in particular (p < 0.001). Inter-reader agreement of AFs favoring benignity was poor to moderate (k range: - 0.01, 0.43). CONCLUSIONS: AFs favoring benignity are not uncommon in LR-5 observations, but category downgrade is exceptional. KEY POINTS: • Ancillary features favoring benignity are encountered in 5.6-12.3% of observations categorized as LR-5. • Category downgrade of LR-5 observations is very rare (0.6% of observations) in the presence of AFs favoring benignity due to the high prevalence (98-99%) of ancillary features favoring malignancy in LR-5 observations. • The inter-reader agreement of ancillary features favoring benignity is poor to moderate (k range: - 0.01, 0.43) in readers with different levels of experience.

Choline PET/CT features to predict survival outcome in high-risk prostate cancer restaging: a preliminary machine-learning radiomics study.

BACKGROUND: Radiomic features are increasingly utilized to evaluate tumor heterogeneity in PET imaging but to date its role has not been investigated for Cho-PET in prostate cancer. The potential application of radiomics features analysis using a machine-learning radiomics algorithm was evaluated to select 18F-Cho PET/CT imaging features to predict disease progression in PCa. METHODS: We retrospectively analyzed high-risk PCa patients who underwent restaging 18F-Cho PET/CT from November 2013 to May 2018. 18F-Cho PET/CT studies and related structures containing volumetric segmentations were imported in the "CGITA" toolbox to extract imaging features from each lesion. A Machine-learning model has been adapted using NCA for feature selection, while DA was used as a method for feature classification and performance analysis. RESULTS: One hundred and six imaging features were extracted for 46 lesions for a total of 4876 features analyzed. No significant differences between the training and validating sets in terms of age, sex, PSA values, lesion location and size (P>0.05) were demonstrated by the machine-learning model. Thirteen features were able to discriminate FU disease status after NCA selection. Best performance in DA classification was obtained using the combination of the 13 selected features (sensitivity 74%, specificity 58% and accuracy 66%) compared to the use of all features (sensitivity 40%, specificity 52%, and accuracy 51%). Per-site performance of the 13 selected features in DA classification were as follows: T = sensitivity 63%, specificity 83%, accuracy 71%; N = sensitivity 87%, specificity 91% of and accuracy 90%; bone-M = sensitivity 33%, specificity 77% and accuracy 66%. CONCLUSIONS: An artificial intelligence model demonstrated to be feasible and able to select a panel of 18F-Cho PET/CT features with valuable association with PCa patients' outcome.

Prospective cardiac magnetic resonance imaging survey in myelodysplastic syndrome patients: insights from an Italian network.

We prospectively evaluated changes in cardiac and hepatic iron overload (IO) and in morpho-functional cardiac parameters and myocardial fibrosis by magnetic resonance imaging (MRI) in patients with low-risk and intermediate-1-risk myelodysplastic syndromes (MDS). Fifty patients enrolled in the Myocardial Iron Overload in MyElodysplastic Diseases (MIOMED) study were followed for 12 months. IO was quantified by the T2* technique and biventricular function parameters by cine images. Macroscopic myocardial fibrosis was detected by late gadolinium enhancement technique. Twenty-eight patients (71.89±8.46 years; 8 females) performed baseline and follow-up MRIs. Thirteen patients had baseline hepatic IO, with a higher frequency among transfusion-dependent patients. Out of the 15 patients with a baseline MRI liver iron concentration <3 mg/g/dw, two (non-chelated) developed hepatic IO. Thirteen (46.4%) patients had an abnormal T2* value in at least one myocardial segment. One patient without hepatic IO and non-transfused had baseline global T2* <20 ms. Among the 15 patients with no baseline myocardial IO (MIO), 2 worsened. There was a significant increase in both left and right ventricular end-diastolic volume indexes. Thirty-six percent of patients showed myocardial fibrosis correlating with aging. Two new occurrences were detected at the follow-up. In conclusion, by a more sensitive segmental approach, MIO is quite frequent in MDS patients and it can be present also in non-transfused patients and in absence of detectable hepatic iron. The incidence of cardiac and hepatic IO and of myocardial fibrosis and the increase in biventricular volumes after a 12-month interval suggest performing periodic MRI scans to better manage MDS patients.

Long-term evolution of LI-RADS observations in HCV-related cirrhosis treated with direct-acting antivirals.

BACKGROUND & AIMS: The risk of progression of indeterminate observations to hepatocellular carcinoma (HCC) after direct-acting antivirals (DAA) is still undetermined. To assess whether DAA therapy changes the risk of progression of observations with low (LR-2), intermediate (LR-3) and high (LR-4) probability for HCC in cirrhotic patients and to identify predictors of progression. METHODS: This retrospective study included cirrhotic patients treated with DAA who achieved sustained virological response between 2015 and 2019. A total of 68 patients had pre-DAA indeterminate observations and at least six months CT/MRI follow-up before and after DAA. Two radiologists reviewed CT/MRI studies to categorize observations according to the LI-RADSv2018 and assess the evolution on subsequent follow-ups. Predictors of evolutions were evaluated by using the Cox proportional hazard model, Kaplan-Meier method and log-rank test. RESULTS: A total of 109 untreated observations were evaluated, including 31 (28.4%) LR-2, 67 (61.5%) LR-3 and 11 (10.1%) LR-4. During a median follow-up of 41 months, 17.4% and 13.3% of observations evolved to LR-5 or LR-M and LR-5, before and after DAA respectively (P = .428). There was no difference in rate of progression of neither LR-2 (P = 1.000), LR-3 (P = .833) or LR-4 (P = .505). At multivariate analysis, only initial LI-RADS category was an independent predictor of progression to LR-5 or LR-M for all observations (hazard ratio 6.75, P < .001), and of progression to LR-5 after DAA (hazard ratio 4.34, P = .047). CONCLUSIONS: DAA therapy does not increase progression of indeterminate observations to malignant categories. The initial LI-RADS category is an independent predictor of observations upgrade.

Radiomics analysis of 18F-Choline PET/CT in the prediction of disease outcome in high-risk prostate cancer: an explorative study on machine learning feature classification in 94 patients.

OBJECTIVE: The aim of this study was (1) to investigate the application of texture analysis of choline PET/CT images in prostate cancer (PCa) patients and (2) to propose a machine-learning radiomics model able to select PET features predictive of disease progression in PCa patients with a same high-risk class at restaging. MATERIAL AND METHODS: Ninety-four high-risk PCa patients who underwent restaging Cho-PET/CT were analyzed. Follow-up data were recorded for a minimum of 13 months after the PET/CT scan. PET images were imported in LIFEx toolbox to extract 51 features from each lesion. A statistical system based on correlation matrix and point-biserial-correlation coefficient has been implemented for features reduction and selection, while Discriminant analysis (DA) was used as a method for features classification in a whole sample and sub-groups for primary tumor or local relapse (T), nodal disease (N), and metastatic disease (M). RESULTS: In the whole group, 2 feature (HISTO_Entropy_log10; HISTO_Energy_Uniformity) results were able to discriminate the occurrence of disease progression at follow-up, obtaining the best performance in DA classification (sensitivity 47.1%, specificity 76.5%, positive predictive value (PPV) 46.7%, and accuracy 67.6%). In the sub-group analysis, the best performance in DA classification for T was obtained by selecting 3 features (SUVmin; SHAPE_Sphericity; GLCM_Correlation) with a sensitivity of 91.6%, specificity 84.1%, PPV 79.1%, and accuracy 87%; for N by selecting 2 features (HISTO = _Energy Uniformity; GLZLM_SZLGE) with a sensitivity of 68.1%, specificity 91.4%, PPV 83%, and accuracy 82.6%; and for M by selecting 2 features (HISTO_Entropy_log10 - HISTO_Entropy_log2) with a sensitivity 64.4%, specificity 74.6%, PPV 40.6%, and accuracy 72.5%. CONCLUSION: This machine learning model demonstrated to be feasible and useful to select Cho-PET features for T, N, and M with valuable association with high-risk PCa patients' outcomes. KEY POINTS: • Artificial intelligence applications are feasible and useful to select Cho-PET features. • Our model demonstrated the presence of specific features for T, N, and M with valuable association with high-risk PCa patients' outcomes. • Further prospective studies are necessary to confirm our results and to develop the application of artificial intelligence in PET imaging of PCa.

Transcranial magnetic resonance-guided focused ultrasound thalamotomy as a safe treatment option in multiple sclerosis patients with essential tremor.

Transcranial magnetic resonance-guided focused ultrasound is a recently introduced incisionless treating option for essential tremor and tremor-dominant idiopathic Parkinson disease. There is preliminary evidence that it may result in a promising effective treatment option for other movement disorders too. Here, we report on two patients with multiple sclerosis with medication refractory debilitating essential tremor comorbidity who successfully underwent unilateral Vim tcMRgFUS thalamotomy for tremor control. Patients' clinical condition and expanded disability status scale scores showed no changes during the 1-year follow-up period with no evidence of multiple sclerosis activity or progression.

Caustic ingestion: CT findings of esophageal injuries and thoracic complications.

Ingestion of caustic substances, whether accidental or for the purpose of suicide, can cause severe lesions of the lips, oral cavity, pharynx, upper gastrointestinal system, and upper airways. In particular, caustic agents could be responsible for severe esophageal injuries resulting in short- and long-term complications. Because of these important clinical implications, timely diagnosis and appropriate management are crucial. In the evaluation of esophageal injuries, thoraco-abdominal computed tomography (CT) is preferable to endoscopy as it avoids the risk of esophageal perforation and allows the evaluation of esophageal injuries as well as of the surrounding tissue. In this review, we report CT findings of esophageal injuries and possible related thoracic complications caused by caustic ingestion.

Whole-body magnetic resonance imaging (WB-MRI) in oncology: an Italian survey.

PURPOSE: To perform a survey among all members of the Italian Society of Medical and Interventional Radiology (SIRM) to assess how whole-body MRI (WB-MRI) is performed in oncologic patients in Italy. METHODS: On March 2019, we administered an online poll to all SIRM members about their use of WB-MRI in 2018 asking 15 questions regarding oncologic indications, imaging protocol, use of contrast media, experience in WB-MRI, duration of scan time and reporting time. RESULTS: Forty-eight members participated to the survey. WB-MRIs/total MRIs ratio was 1%. Lymphoma was the most common indication (17/48, 35%), followed by myeloma and prostate cancer, with these three tumors representing the most common indication in 39/48 of cases (81%). WB-MRI acquisition time and reporting time were 46-60 min in 22/48 centers (46%) and 20-30 min in 19/48 (40%), respectively. WB-MRIs were mostly performed in 1.5T scanners (43/48, 90%), with surface coils (22/48, 46%) being preferred to Q-body (15/48, 31%) and integrated coils (11/48, 23%). Contrast media were injected in 22/48 of the centers (46%), mainly used for breast cancer (13/22, 59%). DWI was the most used sequence (45/48, 94%), mostly with b800 (27/48, 56%), b0 (24/48, 50%) and b1000 (20/48, 42%) values. In about half of cases, radiologists started evaluating WB-MRI non-contrast morphologic sequences, then checking DWI and post-contrast images. CONCLUSION: WB-MRI was mainly performed at 1.5T unit, with lymphoma, myeloma and prostate cancer having been the most common indications. The extreme variability in the choice of imaging protocols and use of contrast agents demonstrates the need of a standardization of WB-MRI application in clinical practice.

Sicilian Byzantine Icons through the Use of Non-Invasive Imaging Techniques and Optical Spectroscopy: The Case of the Madonna dell'Elemosina.

The iconographic heritage is one of the treasures of Byzantine art that have enriched the south of Italy, and Sicily in particular, since the early 16th century. In this work, the investigations of a Sicilian Icon of Greek-Byzantine origin, the Madonna dell'Elemosina, is reported for the first time. The study was carried out using mainly non-invasive imaging techniques (photography in reflectance and grazing visible light, UV fluorescence, infrared reflectography, radiography, and computed tomography) and spectroscopic techniques (X-ray fluorescence and infrared spectroscopy). The identification of the constituent materials provides a decisive contribution to the correct historical and artistic placement of the Icon, a treasure of the Eastern European historical community in Sicily. Some hidden details have also been highlighted. Most importantly, the information obtained enables us to define its conservation state, the presence of foreign materials, and to direct its protection and restoration.